In the name of God Case presentation PatientFemale DOB 91095 29122016 Age 5MO Second child parents First degree 37 weeks HC 36cm 4400 gr ID: 935933
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Slide1
Case presentationImmune Hemolytic Anemia
In the name of God
Slide2Case presentation
Patient:Female
DOB :
9/10/95
( 29/12/2016
)
Age: 5MO
Second child ,
parents: First degree
, 37 weeks, HC: 36cm, 4400
gr
,
C/S duo to decrease of fetal movement
First Child : OK
Admission :Amir kola Hospital :9/10/95
CC & PE:
Dyspnea
, Cyanosis,
Hepatosplenomegaly
, Edema ,Decrease O2 saturation (So2 < 30%)
VBG: PH: 7.05 PCO2: 79 SO2 : 41% HCO3 : 22
Slide3Work up
Impression :
CHD and PFC
Diagnosis:Hydrops
Fetalis
- Non immune
First CBC:
WBC: 22300
RBC: 1.33
Hb
: 4.5
MCV:
112
MCH : 33 MCHC: 30 Plate :
85000
Retic
: 1.4 % D-Coombs:
neg
SGOT : 38 SG PT : 28 Alb: 2
BilT
/D:2.3 / 0.3
G6PD : sufficient CRP : 14 high (135 , 68)
BG neonate : O- Mother A+
CXR:Cardiomegaly
ECHO: RVE , LVE, TR, MR
Abdo
Sonography
: large liver , spleen
Nl
11/10/95
Slide4Work upAnti
ParvoVirus
:
Neg
22/10 /95
(PCR PVB19 :???)
Torch study & metabolic disorders
Management:
Intubation , Ventilation, AB-therapy , Surfactant & PG ,
Milrinon
,
Isovolume
Exchange 165 cc ( 9/10/95
) ,
packed cell transfusion ( 10/10 45cc +
lasix
)
Discharge ; 26/10/95 good condition
Slide5Follow Up in AMIR KOLA HOSPITAL
Follow Up :
1. 5 mo old ; visit : Anemia
Recommendation EP ,(
Hb
:7) At first good response ,(
Hb
10)
Again anemia
:
7/2/96
Admisson
& visit of Hematologist
WBC: 13000
RBc
: 2.06 0.000
Hb
: 6 MCV : 86
MCh
: 29 MCHC: 33
Plate :
Nl
Retic
: 0.5 % Coombs :
neg
7/2/96 PC transfusion
Mother;RBC
:5.800000
Hb
:13.7 MCV : 71 MCH : 23
HbE
:
Nl
A2 2.5 % A; 97.5
Father: RBC: 4.45
Hb
: 14 MCV : 88 NL
Hb
: NL
Erythropoietin level:116 high(9/2/96)
Hb
E patient : HB
Barts
: 43%??? HBA:50% HbA2:1.9% HbF;4.25% (13/2/96)
Slide6Follow Up in AMIR KOLA HOSPITAL
16/2/96 :
HB: 6
Coombs : 1+ pos
PB19 Mother :
Neg
Infant PV B19 IgG;0.5
IgM
: 2.3pos
(11/2/96)
IVIG: Infusion
23/2/96 :
Hb
: 6
Slide7Work up / Mofid Hospital
Admission in
Mofid
hospital 25/2/96 Age :4. 5 mo
PE: good baby ,
mild
icter
in sclera,
Hepatomehgaly
, pallor
WBC : 23300 N ; 51 % L ; 43
RBC: 1.800,000
Hb
: 5
MCV: 73 MCH : 27 Plate : 540000 RDW:17
Retic
; <0.1 %
Coombs : Direct : 4+ Indirect : 1+
Aniso
, Hypo ,
Poikilo
,
Sper
1-2 +
ALK : 986
Nl
SGOT ; 340 SGPT : 546
LDH : 1156
Urine ;
nl
Coombs D/I ,
Ab
scrennng
mother:
Neg
Antibody
screning
patient ; Tube method : Auto control: Pos 4+
Strong Warm
AutoAb
IgG
; 3+ Complement C3D : positive : 1+
Drugs : IVIG 1gr/kg x 4 days &
Cortico
(CS)
Slide8Work up / Mofid Hospital
CXR
ECHO :
Nl
Abd
Sono
: Liver : 75mm,liver span increase Spleen :
Nl
size
BMA : Increased
Cellularity
in M & E series
28/2/96
Slide9Bone Marrow
Slide10Bone Marrow
Slide11Bone Marrow
Slide12Work up / Mofid Hospital
Virology :
Toxo
(Patient) :
IgG
,
IgM
+( mild
incease
)
Infectous
consult : No treatment
Eye Exam:
Nl
Brain CT : Benign subarachnoid
enlargment
,
No calcification
EBV & CMV:
Neg
PCR
Parvovirus B19 PCR: positive
IG:
Nl
-
IgG
increased ( IVIG infusion)
Collagen vascular tests patient & Mother : Negative
Mother Torch :
Neg
Thalassemia
Gene Analysis: final result-Pending
Slide13Follow up / Mofid hospital
Duo to progressive rising
of
Retic
to 14-15%
&
high level of LFT and Gamma GT: 193
GI consult : Immune , Viral , Giant cell hepatitis
Biopsy of Liver was done
Puls
MP and 1
gr
/kg
IvIg
and
Azathioperine
2mg/kg /day/
po
Duo to decrease
Hb
to 6.5 and ,
persistant
high
Retic;
Rituxmab
:375mg/m2 / Iv infusion x 4weeks
Slide14Mofid hospital/ Lab tests
14/3
11/3
9/3
7/3
6/3
3/3
2/3
29/2
28/2
26/2
26/2
Date
8.6
9
9.6
9.9
11
6.9
7.8
11.4
6.5
10
5
Hb
93
87
90
87
84
81
79
78
73
MCV
10%
15%
13%
-
11 %
14%
8%
7%
<0.1
<0.5
Retic
104
131
235
103
156
488
340
SGOT
245
253
545
262
378
271
546
SGPT
2.8/
1.2
2.8/
0.5
5.5/
0.7
3.2/
1.8
3.7/
0.6
2.7/1.3
BilT
/D
900
1800
1151
LDH
Slide15Recent Lab testsLast CBC :20/3/96 after
Rituximab
2
RBC : 2.460.000
Hb
: 8.6
MCV: 95 MCH 34
MCHC: 36
Retic
: 5.5% Coombs: 2+
SGOT :170 SGPT:188
BILT/D: 2.2/0.9
Drugs:
Azathioperinr
and
prednison
( On taper), Acid Folic
pathology Report of liver Biopsy:
Mild Giant Cell transformation ,
Cholestasis
, Mild
microvesicular
steatosis
, and some
asinar
formation,
work up for Metabolic
disese
Slide16PB Smear
Slide17PB Smear
Slide18PB Smear
Slide19PB Smear
Slide20PB Smear
Slide21PB Smear
Slide22Thank You